1 Table of Contents 2
1.1 List of Tables 3
1.2 List of Figures 3

2 Pulmonary Arterial Hypertension: Executive Summary 5
2.1 Related Reports 7
2.2 Upcoming Reports 8

3 Epidemiology 9
3.1 Disease Background 9
3.2 Risk Factors and Comorbidities 11
3.3 Global and Historical Trends 11
3.4 Forecast Methodology 13
3.4.1 Sources 13
3.4.2 Forecast Assumptions and Methods 21
3.4.3 Diagnosed Incident Cases and Diagnosed Prevalent Cases of PAH (Forecast Based on Registry Data) 22
3.4.4 Diagnosed Prevalent Cases of PAH by NYHA Functional Class I-IV, 7MM (Forecast Based on Registry Data) 26
3.4.5 Diagnosed Prevalent Cases with Comorbidities (Forecast Based on Registry Data) 28
3.4.6 Diagnosed Incident Cases and Diagnosed Prevalent Cases - US, France, Germany, Italy, Spain, and UK (Forecast Adjusted for Underestimation of Registry Data) 30
3.4.7 Diagnosed Prevalent Cases by NYHA Functional Class I-IV, US, France, Germany, Italy, Spain, and UK (Forecast Adjusted for Underestimation of Registry Data) 31
3.4.8 Diagnosed Prevalent Cases by Comorbidities (Adjusted for Underestimation) 31
3.5 Epidemiological Forecast for PAH (2016-2026) - Based on Registry Data 31
3.5.1 Diagnosed Incident Cases of PAH (Forecast Based on Registry Data) 31
3.5.2 Diagnosed Prevalent Cases of PAH (Forecast Based on Registry Data) 32
3.5.3 Diagnosed Prevalent Cases of PAH by NYHA Functional Classes (Forecast Based on Registry Data) 33
3.5.4 Comorbidities Among the Diagnosed Prevalent Cases of PAH (Forecast Based on Registry Data) 34
3.6 Epidemiological Forecast for PAH (2016-2026) - Adjusted for Underestimation of Registry Data 34
3.6.1 Diagnosed Incident Cases of PAH (Forecast Adjusted for Underestimation of Registry Data) 34
3.6.2 Age-Specific Diagnosed Incident Cases of PAH (Forecast Adjusted for Underestimation of Registry Data) 35
3.6.3 Sex-Specific Diagnosed Incident Cases of PAH (Forecast Adjusted for Underestimation of Registry Data) 37
3.6.4 Diagnosed Prevalent Cases of PAH (Forecast Adjusted for Underestimation of Registry Data) 37
3.6.5 Age-Specific Diagnosed Prevalent Cases of PAH (Forecast Adjusted for Underestimation of Registry Data) 38
3.6.6 Sex-Specific Diagnosed Prevalent Cases of PAH (Forecast Adjusted for Underestimation of Registry Data) 39
3.6.7 Diagnosed Prevalent Cases of PAH by NYHA Functional Class I-IV (Forecast Adjusted for Underestimation of Registry Data) 40
3.6.8 Comorbidities Among the Diagnosed Prevalent Cases of PAH (Forecast Adjusted for Underestimation of Registry Data) 41
3.7 Discussion 42
3.7.1 Epidemiological Forecast Insight 42
3.7.2 Limitations of Analysis 43
3.7.3 Strengths of Analysis 43

4 Appendix 45
4.1 Bibliography 45
4.2 About the Authors 48
4.2.1 Epidemiologist 48
4.2.2 Reviewers 48
4.2.3 Global Director of Therapy Analysis and Epidemiology 49
4.2.4 Global Head and EVP of Healthcare Operations and Strategy 49
4.3 About GlobalData 50
4.4 Contact Us 50
4.5 Disclaimer 50

1.1 List of Tables
Table 1: WHO Guidelines, Modified NYHA Functional Classes I-IV 10
Table 2: Risk Factors and Comorbidities for PAH 11
Table 3: 7MM, Diagnosed Incident Cases of PAH, Both Sexes, All Ages, N, Selected Years 2016-2026 (Based on Registry Data) 32
Table 4: 7MM, Diagnosed Prevalent Cases of PAH, Both Sexes, All Ages, N, Selected Years 2016-2026 (Forecast Based on Registry Data). 33
Table 5: 7MM, Diagnosed Incident Cases of PAH, Both Sexes, All Ages, N, Selected Years 2016-2026 (Forecast Adjusted for Underestimation of Registry Data) 35
Table 6: 7MM, Diagnosed Prevalent Cases of PAH, Both Sexes, All Ages, N, Selected Years 2016-2026 (Forecast Adjusted for Underestimation of Registry Data) 38

1.2 List of Figures
Figure 1: 7MM, Diagnosed Incident Cases of PAH, Both Sexes, All Ages, N, 2016 and 2026 (Forecast Adjusted for Underestimation of Registry Data) 6
Figure 2: 7MM, Diagnosed Prevalent Cases of PAH, Both Sexes, All Ages, N, 2016 and 2026 (Forecast Adjusted for Underestimation of Registry Data) 7
Figure 3: 7MM, Age-Standardized Diagnosed Incidence of PAH (Cases per 100,000 Population), All Ages, by Sex, 2016 (Forecast Based on Registry Data) 12
Figure 4: 7MM, Age-Standardized Diagnosed Prevalence of PAH (%), All Ages, by Sex, 2016 (Forecast Based on Registry Data) 13
Figure 5: 7MM, Sources Used and Not Used for the Diagnosed Incident Cases of PAH and Diagnosed Prevalent Cases of PAH (for Forecast Based on Registry Data and Forecast Adjusted for Underestimation of Registry Data) 15
Figure 6: 7MM, Sources Used for the Diagnosed Prevalent Cases of PAH* by NYHA Functional Classes I-IV (for Forecast Based on Registry Data and Forecast Adjusted for Underestimation of Registry Data) 16
Figure 7: 7MM, Sources Used for the Diagnosed Prevalent Cases of PAH with CTD (for Forecast Based on Registry Data and Forecast Adjusted for Underestimation of Registry Data) 17
Figure 8: 7MM, Sources Used for the Diagnosed Prevalent Cases of PAH with SS (for Forecast Based on Registry Data and Forecast Adjusted for Underestimation of Registry Data) 18
Figure 9: 7MM, Sources Used for the Diagnosed Prevalent Cases of PAH with Diabetes (for Forecast Based on Registry Data and Forecast Adjusted for Underestimation of Registry Data) 19
Figure 10: 7MM, Sources Used for the Diagnosed Prevalent Cases of PAH with COPD (for Forecast Based on Registry Data and Forecast Adjusted for Underestimation of Registry Data) 19
Figure 11: 7MM, Sources Used for the Diagnosed Prevalent Cases of PAH with PT (for Forecast Based on Registry Data and Forecast Adjusted for Underestimation of Registry Data) 20
Figure 12: 7MM, Sources Used for the Diagnosed Prevalent Cases of PAH with CHD (for Forecast Based on Registry Data and Forecast Adjusted for Underestimation of Registry Data) 21
Figure 13: 7MM, Diagnosed Prevalent Cases of PAH, by NYHA Functional Class I-IV, Both Sexes, All Ages, N, 2016 (Forecast Based on Registry Data) 33
Figure 14: 7MM, Comorbidities Among the Diagnosed Prevalent Cases of PAH (N), All Ages, 2016 (Forecast Based on Registry Data) 34
Figure 15: 7MM, Age-Specific Diagnosed Incident Cases of PAH, Both Sexes, All Ages, N, 2016 (Forecast Adjusted for Underestimation of Registry Data) 36
Figure 16: 7MM, Sex-Specific Diagnosed Incident Cases of PAH, All Ages, N, 2016 (Forecast Adjusted for Underestimation of Registry Data) 37
Figure 17: 7MM, Age-Specific Diagnosed Prevalent Cases of PAH, Both Sexes, All Ages, N, 2016 (Forecast Adjusted for Underestimation of Registry Data) 39
Figure 18: 7MM, Sex-Specific Diagnosed Prevalent Cases of PAH, All Ages, 2016 (Forecast Adjusted for Underestimation of Registry Data) 40
Figure 19: 7MM, Diagnosed Prevalent Cases of PAH Categorized into NYHA Functional Class I-IV (N), Both Sexes, All Ages, 2016 (Forecast Adjusted for Underestimation of Registry Data) 41
Figure 20: 7MM, Comorbidities Among the Diagnosed Prevalent Cases of PAH (N), Both Sexes, All Ages, 2016 (Forecast Adjusted for Underestimation of Registry Data) 42